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https://www.readbyqxmd.com/read/28453909/cardiotoxicity-and-cardiomyopathy-in-children-and-young-adult-survivors-of-hematopoietic-stem-cell-transplant
#1
REVIEW
Seth J Rotz, Thomas D Ryan, Joel Hlavaty, Stephen A George, Javier El-Bietar, Christopher E Dandoy
Cardiomyopathy is common in long-term survivors of pediatric hematopoietic stem cell transplant (HSCT). Events occurring before and after HSCT when combined with specific insults during HSCT likely contribute to long-term risk. Strategies for detecting subclinical cardiomyopathy prior to patients developing overt heart failure are under investigation. Changes in HSCT preparative regimens and cardioprotective medications administered during chemotherapy may alter the risk for cardiomyopathy. Interventions in long-term survivors such as lifestyle modification and cardioactive medications are of increasing importance...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#2
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28424442/clinical-improvement-and-no-further-need-of-transplant-after-closure-of-pda-with-transcatheter-approach-in-an-end-stage-heart-failure-patient-with-hypertrabeculation
#3
Serhat Koca, Feyza Ayşenur Paç, Ajda Mutlu Mıhçıoğlu, Vedat Kavurt, Denizhan Bağrul
Although heart failure is managed medically most of the time, heart transplantation is still last resort for selected end-stage heart failure patients with noncompaction cardiomyopathy. Presently described for the first time is case of pediatric patient with noncompaction cardiomyopathy who was initially referred to our hospital for heart transplant but underwent PDA repair and improved clinically without need for heart transplant.
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28424438/using-cardiovascular-imaging-modalities-to-determine-cardiac-disorders-before-starting-sports-activities
#4
Özgür Ceylan, Timur Meşe, Meşe Hazım Gürsu
OBJECTIVE: We re-examined children who had previously been declared eligible to participate in competitive sports activities for cardiac disorders, using cardiac investigation protocol. METHODS: Total of 250 children (224 males [89.6%], and 26 females [10.4%]) between the ages of 8 and 17 years who had just started or were already engaged in sports activities were included in the study. Participants had detailed physical examination evaluated by a pediatric cardiologist...
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28401251/delayed-myocardial-enhancement-in-pediatric-hypertrophic-cardiomyopathy-correlation-with-lv-function-echocardiography-and-demographic-parameters
#5
Sonia El Saiedi, Noha Hossam Behairy, Ahmed Kharabish, Reem Esmail, Zeinab Salah Seliem, Mervat Shafik, Wesam El Mozy
Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI)...
April 11, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28382424/plasma-exchange-for-the-patients-with-dilated-cardiomyopathy-in-children-is-safe-and-effective-in-improving-both-cardiac-function-and-daily-activities
#6
Takeshi Moriguchi, Keiichi Koizumi, Kenichi Matsuda, Norikazu Harii, Junko Goto, Daiki Harada, Hisanori Sugawara, Minako Hoshiai, Hiroaki Kise, Akiyasu Baba
Autoantibodies against cardiac proteins play an important role in the development of dilated cardiomyopathy (DCM). The efficacy and safety of apheresis such as immunoadsorption (IA) or plasma exchange (PE) to remove such antibodies have been reported in adult DCM patients. However, apheresis for pediatric DCM has not been performed because of technical difficulty due to relatively low blood volume and instability of hemodynamics. As we have experiences of preforming apheresis on hemodynamically unstable children, we have preformed ten courses of PE on seven child DCM patients including both patients in chronic and acute phase to assess the safety and efficacy to PE...
April 5, 2017: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
https://www.readbyqxmd.com/read/28378408/cancer-recurrence-and-mortality-after-pediatric-heart-transplantation-for-anthracycline-cardiomyopathy-a-report-from-the-pediatric-heart-transplant-study-phts-group
#7
Matthew J Bock, Elfriede Pahl, Paolo G Rusconi, Gerard J Boyle, John J Parent, Clare J Twist, James K Kirklin, Elizabeth Pruitt, Daniel Bernstein
We aimed to determine whether malignancy after pediatric HTx for ACM affects overall post-HTx survival. Patients <18y listed for HTx for ACM in the PHTS database between 1993 and 2014 were compared to those with DCM. A 2:1 matched DCM cohort was also compared. Wait-list and post-HTx survival, along with freedom from common HTx complications, were compared. Eighty subjects were listed due to ACM, whereas 1985 were listed for DCM. Although wait-list survival was higher in the ACM group, post-HTx survival was lower for the ACM cohort...
April 4, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28374048/lone-pediatric-atrial-fibrillation-in-the-united-states-analysis-of-over-1500-cases
#8
Iqbal El-Assaad, Sadeer G Al-Kindi, Elizabeth V Saarel, Peter F Aziz
Little is known about lone atrial fibrillation (AF) in pediatrics and its risk factors due to low prevalence. We sought to determine risk factors and estimate recurrence rates in children with lone AF using a large clinical database. Using the Explorys clinical database, we retrospectively identified patients who were below 20 years of age at the time of their AF diagnosis. Patients with congenital heart disease, cardiomyopathy, prior open heart surgery, or thyroid disease were excluded. Out of 7,969,230 children identified, 1910 had AF and 1570 met the definition of lone AF...
April 3, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28369760/clinical-genetic-testing-in-pediatric-cardiomyopathy-is-bigger-better
#9
Allison Chang Ouellette, Jacob Mathew, Ashok Kumar Manickaraj, George Manase, Laura Zahavich, Judith Wilson, Kristen George, Lee Benson, Sarah Bowdin, Seema Mital
For clinical genetic testing of cardiomyopathy (CMP), current guidelines do not address which gene panels to use: targeted panels specific to a CMP phenotype or expanded (panCMP) panels that include genes associated with multiple phenotypic subtypes. Our objective was to assess the clinical utility of targeted versus panCMP panel testing in pediatric CMPs. 151 pediatric patients with primary hypertrophic (n = 66), dilated (n = 64), restrictive (n = 8), or left-ventricular non-compaction (n = 13) CMP who underwent clinical genetic panel testing at a single centre were included...
March 30, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28363739/functional-status-of-united-states-children-supported-with-a-left-ventricular-assist-device-at-heart-transplantation
#10
Anica Bulic, Katsuhide Maeda, Yulin Zhang, Sharon Chen, Doff B McElhinney, John C Dykes, Amanda M Hollander, Seth A Hollander, Jenna Murray, Olaf Reinhartz, Mary Alice Gowan, David N Rosenthal, Christopher S Almond
BACKGROUND: As survival with pediatric left ventricular assist devices (LVADs) has improved, decisions regarding the optimal support strategy may depend more on quality of life and functional status (FS) rather than mortality alone. Limited data are available regarding the FS of children supported with LVADs. We sought to compare the FS of children supported with LVADs vs vasoactive infusions to inform decision making around support strategies. METHODS: Organ Procurement and Transplant Network data were used to identify all United States children aged between 1 and 21 years at heart transplant (HT) between 2006 and 2015 for dilated cardiomyopathy and supported with an LVAD or vasoactive infusions alone at HT...
March 2, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28357450/an-alternative-strategy-for-bridge-to-transplant-recovery-in-small-children-with-dilated-cardiomyopathy
#11
REVIEW
Gilles Mets, Joseph Panzer, Daniël De Wolf, Thierry Bové
Dilated cardiomyopathy in children still has a poor prognosis with high rates of mortality and cardiac transplantation (resp. around 20 and 25%). Awaiting transplantation or possible recovery, these pediatric patients are mechanically supported with extracorporeal membrane oxygenation or a paracorporeal ventricular assist device, both resulting in higher survival rates but also entailing considerable risks of infection, thrombosis, or bleeding. A new indication for an old technique, i.e., pulmonary artery banding, presents itself as an interesting alternative to mechanical circulatory support in selected infants and small children with dilated LV cardiomyopathy and preserved RV function...
March 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28325512/-sudden-death-of-a-young-man-as-late-sequelae-complicating-a-pediatric-disease-about-a-case
#12
Clémence Pierry, Camille Franchet, Lucile Tuchtan-Torrents, Céline Macon, Julia Torrents, Caroline Capuani, Marie-Dominique Piercecchi-Marti
In France, sudden death is responsible every year for 40,000 deaths. The most frequent etiology is cardiac disease. Atheromatous-related pathology is the most common etiology beyond 35, but cardiomyopathies and channelopathies are responsible for a significant number of deaths in young adults. Some acquired disorders can also cause sudden cardiac death. We report the case of a 17-year-old man who died suddenly after sport. Autopsy and pathological study found multiple giant coronary aneurysms. Thrombosis and fibrous scar of myocardial ischemic events were observed...
April 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28315942/forgotten-right-ventricle-in-pediatric-dilated-cardiomyopathy
#13
Hala Mounir Agha, Hossam Ibrahim, Inas Abd El Satar, Naglae Abd El Rahman, Doaa Abd El Aziz, Zeinab Salah, Sonia El Saeidi, Fatma Mostafa, Wael Attia, Mohamed Abd El Rahman, Gaser Abd El Mohsen
To evaluate the right ventricular (RV) function in relation to that of the left ventricle (LV) in patients with dilated cardiomyopathy (DCM). Echocardiographic examination was done using tissue Doppler imaging (TDI) and two-dimensional speckle tracking echocardiography (2D-STE) for 32 pediatric patients with DCM comparing them to another 32 normal matched controls. The global longitudinal strain (GLS) derived from 2D-STE was used to reflect the LV systolic function. Tricuspid annular plan systolic excursion (TAPSE) and the following RV TDI derived indexes: peak systolic velocity (S'), peak early diastolic velocity E', peak late diastolic velocity A', isovolumic acceleration (IVA) and myocardial performance index (MPI) were measured...
March 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28295963/a-multicenter-study-of-the-impella-device-for-mechanical-support-of-the-systemic-circulation-in-pediatric-and-adolescent-patients
#14
V Vivian Dimas, Brian H Morray, Dennis W Kim, Christopher Sd Almond, Shabana Shahanavaz, Sebastian C Tume, Lynn F Peng, Doff B McElhinney, Henri Justino
OBJECTIVES: The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age ≤ 21 yrs). BACKGROUND: Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series. METHODS: This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions...
March 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28295041/exome-sequencing-identifies-primary-carnitine-deficiency-in-a-family-with-cardiomyopathy-and-sudden-death
#15
Najim Lahrouchi, Elisabeth M Lodder, Maria Mansouri, Rafik Tadros, Layla Zniber, Najlae Adadi, Sally-Ann B Clur, Karin Y van Spaendonck-Zwarts, Alex V Postma, Abdelaziz Sefiani, Ilham Ratbi, Connie R Bezzina
Pediatric cardiomyopathy is a rare but severe disease with high morbidity and mortality. The causes are poorly understood and can only be established in one-third of cases. Recent advances in genetic technologies, specifically next-generation sequencing, now allow for the detection of genetic causes of cardiomyopathy in a systematic and unbiased manner. This is particularly important given the large clinical variability among pediatric cardiomyopathy patients and the large number of genes (>100) implicated in the disorder...
March 15, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28290258/parent-reports-of-health-related-quality-of-life-and-heart-failure-severity-score-independently-predict-outcome-in-children-with-dilated-cardiomyopathy
#16
Susanna L den Boer, Sara J Baart, Marijke H van der Meulen, Gabriëlle G van Iperen, Ad P Backx, Arend D Ten Harkel, Lukas A Rammeloo, Gideon J du Marchie Sarvaas, Ronald B Tanke, Willem A Helbing, Elisabeth M Utens, Michiel Dalinghaus
BACKGROUND: Dilated cardiomyopathy in children causes heart failure and has a poor prognosis. Health-related quality of life in this patient group is unknown. Moreover, results may provide detailed information of parents' sense of their child's functioning. We hypothesised that health-related quality of life, as rated by parents, and the paediatric heart failure score, as assessed by physicians, have both predictive value on outcome. Methods and results In this prospective study, health-related quality of life was assessed by parent reports: the Infant Toddler Quality of Life questionnaire (0-4 years) or Child Health Questionnaire-Parent Form 50 (4-18 years) at 3-6-month intervals...
March 14, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28289528/clinical-cardiac-regenerative-studies-in-children
#17
REVIEW
Imre J Pavo, Ina Michel-Behnke
Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients...
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28260473/perioperative-venoarterial-extracorporeal-membrane-oxygenation-support-during-heart-transplant
#18
Ender Gedik, Funda Atar, Aycan Ozdemirkan, Aynur Camkiran Firat, Pinar Zeyneloglu, Atilla Sezgin, Arash Pirat
OBJECTIVES: Heart transplant is the only definitive treatment of end-stage heart failure. Venoarterial extracorporeal membrane oxygenation may be used as a bridge to heart transplant. This technique may be used after heart transplant for conditions refractory to medical treatment like primary graft failure. Previously, we reported our experience with patients who received extracorporeal support as a bridge to emergency heart transplant. In this study, we present our perioperative experience with heart transplants in which extracorporeal support was used...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28258649/the-cardiac-manifestations-of-inherited-metabolic-diseases-in-children
#19
REVIEW
David F A Lloyd, Roshni Vara, Sujeev Mathur
Inborn errors of metabolism (IEMs) are responsible for around 5% of all cases of cardiomyopathy (CM) and 15% of non-idiopathic cases. Storage disorders such as Pompe disease (glycogen storage disease type II) typically cause hypertrophic cardiomyopathy, whereas the accumulation of toxic metabolites, as seen in the organic acidurias, is associated with dilated cardiomyopathy (DCM). Mixed pathology is also possible, particularly in late presentations. IEMs such as Barth syndrome, a disorder of cardiolipin stability usually associated with DCM, have been associated with rarer types of CM such as endocardial fibroelastosis (EFE) and left ventricular non-compaction...
March 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28258593/hypertrophic-cardiomyopathy-in-youth-athletes-successful-screening-with-point-of-care-ultrasound-by-medical-students
#20
John C Fox, Shadi Lahham, Graciela Maldonado, Suzi Klaus, Bassil Aish, Lauren V Sylwanowicz, Justin Yanuck, Sean P Wilson, Mason Shieh, Craig L Anderson, Carter English, Ryan Mayer, Uthara R Mohan
OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is a life-threatening genetic cardiovascular disease that often goes undetected in young athletes. Neither history nor physical examination are reliable to identify those at risk. The objective of this study is to determine whether minimally trained medical student volunteers can use ultrasound to screen for HCM. METHODS: This was a prospective enrollment of young athletes performed at 12 area high schools and three area colleges, between May 2012 and August 2013...
March 4, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
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